PKU dietary handbook to accompany PKU guidelines.

Authors :: MacDonald A
van Wegberg AMJ
Ahring K
Beblo S
Bélanger-Quintana A
Burlina A
Campistol J
Coşkun T
Feillet F
Giżewska M
Huijbregts SC
Leuzzi V
Maillot F
Muntau AC
Rocha JC
Romani C
Trefz F
van Spronsen FJ
Source :: Orphanet journal of rare diseases [Orphanet J Rare Dis] 2020 Jun 30; Vol. 15 (1), pp. 171. Date of Electronic Publication: 2020 Jun 30.
Publication Year :: 2020
Abstract: Background: Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts phenylalanine into tyrosine.<br />Main Body: In 2017 the first European PKU Guidelines were published. These guidelines contained evidence based and/or expert opinion recommendations regarding diagnosis, treatment and care for patients with PKU of all ages. This manuscript is a supplement containing the practical application of the dietary treatment.<br />Conclusion: This handbook can support dietitians, nutritionists and physicians in starting, adjusting and maintaining dietary treatment.